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Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis
Abstract
Background Chronic progressive lung disease is the most prominent cause of morbidity and death in patients with cystic fibrosis (CF), but severity of lung disease and rate of lung function decline are widely variable. Accurate estimates of decline have been difficult to define and compare because the timing of measurements and duration of follow-up differ in various patient groups. Patients Three hundred sixty-six patients with CF, born from 1960 to 1974, were selected from a CF database birth cohort if they had two or more measurements of pulmonary function, at least one of which was performed before the age of 10 years. Methods Mixed model regression analysis provided estimates of the average rate of decline of spirometry measurements in subgroups on the basis of survival age, sex, pancreatic status, and genotype. Results Patients who died before the age of 15 years had significantly poorer pulmonary function when first tested and a more rapid decline in pulmonary function thereafter than patients who survived beyond the age of 15 years. In the latter, functional levels at the age of 5 years were normal, but average rates of decline were significantly related to survival age. Female patients had significantly steeper decline than male patients, and those with pancreatic insufficiency had much steeper decline than those with pancreatic sufficiency. In the subset of 197 who survived to 1990 and were subsequently genotyped, rate of decline was greater in those homozygous for t…
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“More rapid decline is associated with more severe CF lung disease and earlier mortality”
From EFFECT OF ELEXACAFTOR/TEZACAFTOR/IVACAFTOR ON ANNUAL RATE OF LUNG FUNCTION DECLINE IN PEOPLE WITH CYSTIC FIBROSIS (2023) · cites this paper as [3]
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“preserving lung function is a primary goal of CF clinical care [ 2 , 3 ].”
From EFFECT OF ELEXACAFTOR/TEZACAFTOR/IVACAFTOR ON ANNUAL RATE OF LUNG FUNCTION DECLINE IN PEOPLE WITH CYSTIC FIBROSIS (2023) · cites this paper as [3]
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Cited by
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Factors associated with FEV1decline in cystic fibrosis: analysis of the ECFS Patient Registry
Kerem, E., Viviani, L., Zolin, A., MacNeill, S., Hatziagorou, E., Ellemunter, H., Drevinek, P., Gulmans, V., Krivec, U., & Olesen, H. (2014) · European Respiratory Journal · doi:10.1183/09031936.00166412 · Cited by 296
Massive hemoptysis in cystic fibrosis
Flume, P. A., Yankaskas, J. R., Ebeling, M., Hulsey, T., & Clark, L. L. (2005) · Chest · doi:10.1378/chest.128.2.729 · Cited by 215
Pneumothorax in cystic fibrosis
Flume, P. A., Strange, C., Ye, X., Ebeling, M., Hulsey, T., & Clark, L. L. (2005) · Chest · doi:10.1378/chest.128.2.720 · Cited by 164
Lee, T., Sawicki, G. S., Altenburg, J., Millar, S. J., Geiger, J. M., Jennings, M. T., Lou, Y., McGarry, L. J., Van Brunt, K., & Linnemann, R. W. (2023) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2022.12.009 · Cited by 56
Metadata
- Authors
- Corey, M., Edwards, L., Levison, H., & Knowles, M.
- Venue
- The Journal of Pediatrics
- Publisher
- Elsevier BV
- Published
- 1997-12
- Volume
- 131
- Issue
- 6
- Pages
- 809-814
- ISSN
- 0022-3476
- Cited by
- 283
- References
- 1
- Language
- en
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